Molecular pathology of neurodegenerative diseases by cryo-EM of amyloids

SHW Scheres, B Ryskeldi-Falcon, M Goedert - Nature, 2023 - nature.com
Abnormal assembly of tau, α-synuclein, TDP-43 and amyloid-β proteins into amyloid
filaments defines most human neurodegenerative diseases. Genetics provides a direct link …

Creutzfeldt–Jakob disease and other prion diseases

I Zerr, A Ladogana, S Mead, P Hermann… - Nature Reviews …, 2024 - nature.com
Prion diseases share common clinical and pathological characteristics such as spongiform
neuronal degeneration and deposition of an abnormal form of a host-derived protein, termed …

MoDAFold: a strategy for predicting the structure of missense mutant protein based on AlphaFold2 and molecular dynamics

L Zheng, S Shi, X Sun, M Lu, Y Liao… - Briefings in …, 2024 - academic.oup.com
Protein structure prediction is a longstanding issue crucial for identifying new drug targets
and providing a mechanistic understanding of protein functions. To enhance the progress in …

Cryo-EM of prion strains from the same genotype of host identifies conformational determinants

F Hoyt, P Alam, E Artikis, CL Schwartz… - PLoS …, 2022 - journals.plos.org
Prion strains in a given type of mammalian host are distinguished by differences in clinical
presentation, neuropathological lesions, survival time, and characteristics of the infecting …

Prion strains viewed through the lens of cryo-EM

SW Manka, A Wenborn, J Collinge… - Cell and Tissue …, 2023 - Springer
Mammalian prions are lethal transmissible pathogens that cause fatal neurodegenerative
diseases in humans and animals. They consist of fibrils of misfolded, host-encoded prion …

Cryo-EM structures of cotton wool plaques' amyloid β and of tau filaments in dominantly inherited Alzheimer disease

MR Hoq, A Fernandez, FS Vago, GI Hallinan… - Acta …, 2024 - Springer
Cotton wool plaques (CWPs) have been described as features of the neuropathologic
phenotype of dominantly inherited Alzheimer disease (DIAD) caused by some missense and …

Emerging trends in cryo-EM-based structural studies of neuropathological amyloids

S Mishra - Journal of Molecular Biology, 2023 - Elsevier
Tauopathies, synucleinopathies, Aβ amyloidosis, TDP-43 proteinopathies, and prion
diseases-these neurodegenerative diseases have in common the formation of amyloid …

[HTML][HTML] The structural line between prion and “prion-like”: Insights from prion protein and tau

C Glynn, JA Rodriguez, BT Hyman - Current opinion in neurobiology, 2024 - Elsevier
The concept of 'prion-like'behavior has emerged in the study of diseases involving protein
misfolding where fibrillar structures, called amyloids, self-propagate and induce disease in a …

Cross-β helical filaments of tau and TMEM106B in gray and white matter of multiple system tauopathy with presenile dementia

MR Hoq, SR Bharath, GI Hallinan, A Fernandez… - Acta …, 2023 - Springer
In neurodegenerative diseases and aging, the microtubuleassociated protein tau (MAPT)
and the transmembrane lysosomal protein 106B (TMEM106B) become misfolded in different …

Cryo-EM confirms a common fibril fold in the heart of four patients with ATTRwt amyloidosis

BA Nguyen, V Singh, S Afrin, P Singh, M Pekala… - Communications …, 2024 - nature.com
ATTR amyloidosis results from the conversion of transthyretin into amyloid fibrils that deposit
in tissues causing organ failure and death. This conversion is facilitated by mutations in …